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Craniosynostosis

07/01/2022 Conditions

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Overview

Craniosynostosis is a birth defect in which the bones that make up the baby’s skull fuse together too early, before the brain has finished growing.

This premature closure prevents the skull from expanding as the baby’s brain continues to grow.

Craniosynostosis can lead to problems with skull development and brain growth, causing an abnormal head shape and in some cases pressure on the brain. Symptoms can range from mild to severe.

Early diagnosis and treatment are important to achieving the best possible outcomes. Treatment is typically surgery to allow the brain adequate room to develop and to correct the shape of the baby’s head.

While craniosynostosis can occur in combination with other abnormalities, it is usually an isolated condition. Most children with craniosynostosis are otherwise healthy and have normal intelligence.

How does craniosynostosis affect my baby?

A baby’s skull is made up of several bony plates connected at the seams by fibrous material known as “sutures.” These sutures allow the skull to expand as the baby’s brain continues to grow. Normally, around two years of age these bony plates and sutures fuse together to form a solid skull. The fontanels (“soft spots” on a baby’s head) are an example of these sutures and how they fill in with bone over time.

In babies with craniosynostosis, one or more of these sutures fuse together too early, restricting the growth of the skull while the brain underneath that area continues to get bigger. The brain may compensate by growing into a different area in the skull, where the sutures haven’t yet closed and the skull can still expand. This can cause the baby’s head to look misshapen.

If multiple sutures close too early, the brain may not have enough room to grow, putting increasing pressure on the brain.

Untreated, complications of craniosynostosis may include:

  • Worsening head deformity
  • Headaches, vision problems and developmental delays caused by pressure on the brain
  • Abnormal facial features

Cause

Craniosynostosis is a congenital defect, meaning it is present at birth. The cause is typically unknown.

The defect can sometimes occur as part of a genetic disorder with other birth defects.

The condition is estimated to occur in 1 out of every 2,000 births.

Types of Craniosynostosis

There are several types of craniosynostosis, based on the area of the skull (suture) affected. Each type causes the baby’s head to be shaped differently.

Craniosynostosis
  • Sagittal – the most common type, caused by premature fusion of the main suture running along the top of the skull from the front to the back of the head. This type is more common in boys than girls. The head shape is described as scaphocephaly.
  • Coronal (unicoronal or bicoronal) – caused by premature fusion of the sutures that run from each ear to the top of the skull. It is more common in girls. The child’s head shape is described as plagiocephaly.
  • Metopic – a rare form of craniosynostosis caused by premature closure of the metopic (forehead) suture. The metopic suture runs from the top of the head down the middle of the forehead, toward the nose. The child’s head shape is described as trigonocephaly (triangular shaped forehead).
  • Lambdoid – a rare type caused by early closure of the lambdoid suture, which runs across the back of the skull. The head shape is described as posterior plagiocephaly.

Signs and Symptoms

While the signs and symptoms vary with each child, in general they may include:

  • An abnormally shaped head
  • No “soft spot” (fontanelle) or a soft spot that is much smaller than normal
  • A raised, hard ridge where a suture (gap between bones of the skull) closed too early
  • Abnormalities in the shape of the baby’s face
  • Slow growth or no growth in the baby’s head size
  • Unusual sleepiness
  • Irritability that can’t be consoled
  • Poor feeding
  • Seizures
  • Bulging eyes or inability to look upward
  • Developmental delays

Diagnosis

In some cases, craniosynostosis may be diagnosed or suspected during pregnancy following a routine ultrasound, typically in the third trimester.

More often the condition is diagnosed after birth during a physical exam.

Diagnosis should be made by a specialist experienced in this birth defect, such as a pediatric neurosurgeon or plastic surgeon. Imaging tests may be performed to examine the skull and brain in detail, confirm the diagnosis and learn more about the baby’s condition.

It’s important to note that not all abnormal head shapes are a result of craniosynostosis. Accurate diagnosis is critical for proper treatment. Surgery is typically required for craniosynostosis but not for “positional plagiocephaly,” another cause of an abnormally shaped head.

Specialized Evaluation and Care

If craniosynostosis is diagnosed or suspected during pregnancy, you may be referred to a fetal center for further evaluation and specialized care, ensuring proper treatment planning.

At Texas Children’s Fetal Center, we arrange for you to visit as quickly as possible for a comprehensive assessment by a team of specialists experienced in diagnosing and treating craniosynostosis, including maternal-fetal medicine physicians, fetal imaging experts, pediatric neurosurgeons and neurologists, genetic counselors and neonatologists.

You will undergo additional testing to help us confirm the diagnosis and look for other abnormalities.

Our specialists will then meet with you about the results, provide treatment recommendations, and answer any questions your family has, to help you make the most informed decisions regarding your baby’s care and treatment. A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized expertise your baby will need after birth, ensuring timely treatment.

You and your baby will be closely monitored throughout pregnancy with regular ultrasounds to assess fetal growth and watch for signs of complications.

Delivery

A caesarean delivery may be required to minimize risks to the mother and baby caused by skull abnormalities. 

In severe or complex cases, delivery at a center with specialized expertise and resources for the treatment of newborns with craniofacial birth defects may be recommended, including an advanced neonatal intensive care unit (NICU) for premature or critically ill newborns.

Treatment After Birth

Treatment for craniosynostosis is typically surgery to separate the fused bones. The goals of surgery are to allow for normal growth of the brain, relieve the pressure on the brain, and restore or improve the child’s appearance and head shape.

Surgery is generally performed between 2 and 10 months of age by a pediatric neurosurgeon and pediatric plastic surgeon. The timing and type of procedure depends on your child’s condition. In some cases, minimally invasive endoscopic surgery may be an option.

For babies with very mild craniosynostosis, surgery might not be needed.

Postnatal Care Team

Depending on the severity of your baby’s condition, his or her postnatal care team may include:

A unique and distinct advantage for mothers delivering at Texas Children’s Pavilion for Women is our location inside one of the top children’s hospitals in the nation, for seamless access to the critical care services and specialists your child may need after birth.

Our Fetal Center team works closely with Texas Children’s Craniofacial Clinic, a multidisciplinary team of pediatric specialists experienced in the treatment of all types of craniosynostosis.

Why Texas Children’s Fetal Center?

  • A single location for expert maternal, fetal and pediatric care. At Texas Children’s Hospital, you and your baby receive the specialized care required for the diagnosis and treatment of craniosynostosis all in one location, for highly coordinated care and treatment planning.
  • A skilled, experienced team with proven outcomes. We have a dedicated team of maternal-fetal medicine specialists, fetal imaging experts, pediatric neurosurgeons and neurologists, pediatric plastic surgeons, neonatologists and others who work in concert to care for you and your baby every step of the way, using proven protocols we’ve developed over the years. With their combined expertise and unified approach, this team offers the best possible care for babies with craniosynostosis.
  • We care for your child’s needs at every stage of life. Our comprehensive approach starts with your first prenatal visit and continues through delivery, postnatal care, and childhood, thanks to one of the nation’s leading teams of fetal and pediatric specialists for the care and treatment of rare birth defects.

Volumes and Outcomes

Additional Resources

Videos

Research

Texas Children’s, together with our partner institution Baylor College of Medicine, constantly strives to seek new and better treatments for babies with craniofacial abnormalities (abnormalities of the face, jaw and skull).

In 2013, we began using a 3DMD imaging system to assist in the treatment of craniosynostosis patients. This system uses no radiation and only takes a fraction of a second to capture exceptional images that aid in patient evaluation, patient education, surgical planning, and treatment, for the best possible outcomes.

Learn more.

For more information or to schedule an appointment,

call Texas Children’s Fetal Center at 832-822-2229 or 1-877-FetalRx (338-2579) toll-free.

Our phones are answered 24/7. Immediate appointments are often available.